Double-Outlet Right Ventricle

Usually, doctors can diagnose double-outlet right ventricle when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing baby’s heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease.

Your obstetrician may refer you for a fetal echo if your family has a history of congenital heart disease or if a routine prenatal ultrasound shows a problem.

Our Fetal Care and Treatment Center team can care for you when you are pregnant if your developing baby has a known or suspected problem.

To diagnose this condition, your child’s doctor will examine your child, check their heartbeat and pulse and listen to their heart. In children with double-outlet right ventricle, doctors might be able to hear a heart murmur – the sound of blood moving in the heart in a way that’s not normal.

The doctor will ask for details about your child’s symptoms, their health history and your family health history.

Your child will need tests that provide more information about how their heart looks and works. These will most likely include:

• Echocardiography
• Chest X-rays
• Electrocardiography (ECG or EKG)

Your child may also need these tests: 

• MRI (magnetic resonance imaging)
• CT (computed tomography) scan
• Cardiac catheterization

In general, children need surgery to do the following: 

• Direct oxygen-rich blood, which comes from the lungs, into the aorta. From there, it can be carried to the rest of their body.
• Keep oxygen-poor blood, which comes back from the body, out of the aorta. This blood needs to go to your child’s lungs first to pick up oxygen.
• Close any other abnormal holes in the heart. This step keeps oxygen-rich blood from mixing with oxygen-poor blood. 

How doctors achieve these goals depends on the kind of double-outlet right ventricle your child has. The cardiologist and surgeon taking care of your child will talk with you about the best approach for your child’s heart.

Most children have surgery that allows both ventricles to pump blood where it needs to go. For children with some complex defects, the best approach is surgery that allows one ventricle to do all the pumping. The team in our Single Ventricle Program provides specialized care for these children.

All children with double-outlet right ventricle will need lifelong follow-up with a cardiologist who specializes in congenital heart defects.

To get ready for surgery, your child may need medicines like these: 

• Diuretics, which help the kidneys rid the body of extra water
• Angiotensin-converting enzyme (ACE) inhibitors, which lower blood pressure
• Digoxin, which makes the heart beat slower and with more force

In rare cases, the heart cannot be repaired, and doctors may recommend a heart transplant. The heart transplant team at Seattle Children’s is one of the best in the nation, caring for children with this or other heart problems that cannot be controlled using other treatments. Read more about our Heart Transplant Program and see our exceptional outcomes.

• The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many babies with this condition.
• Our doctors and surgeons are experts in the treatments your child may need. These may include surgery to change blood flow in your child’s heart, medicine and a heart transplant.
• Our 4 pediatric cardiac surgeons perform more than 500 procedures yearly. Seattle Children’s surgical outcomes are among the best in the nation year after year.
• We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery. General anesthesia is a medicine we give to people before surgery so they are fully asleep during the procedure.
• The transplant team does several heart transplants each year for children with this or other heart problems that cannot be controlled using other treatments. 
• Your child’s team includes experts from other areas of Seattle Children’s based on their needs, like doctors who specialize in newborns (neonatologists) or lung health (pulmonologists).

• If your developing baby is diagnosed with double-outlet right ventricle before birth, Seattle Children’s Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.
• Your child’s treatment plan is custom-made. We plan and carry out their treatment based on the specific details of their heart defects. We closely check your child’s needs to make sure they get the care that is right for them at every age.
• We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.

• We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
• Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
• Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
• Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make your experience as smooth as possible.
• Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
• Read more about the supportive care we offer.